IgA nephropathy (IgAN) is the most common primary glomerulonephritis (GN) worldwide. Although most IgAN patients have a benign course, the natural history of those with heavy proteinuria is quite variable. Several studies show that corticosteroids (steroids) therapy can decrease proteinuria and stabilize the renal function. Also, angiotensin-converting enzyme inhibitors and angiotensin II type 1 receptor blockers (ARBs) have the benefit of decreasing proteinuria for IgAN patients with and without hypertension. Because heavy proteinuria is an important poor prognostic factor of IgAN, it is worthy to compare the therapeutic responses of corticosteroids, ACEIs/ARBs, and combined therapy of both agents in these patients with heavy proteinuria (i.e. daily protein loss≧3 g). Therefore, a retrospective study of 102 biopsy-proved IgAN patients was conducted in our hospital. We analyzed the clinical features, laboratory data, and managements by reviewing medical records, and 42 patients were excluded, including loss of follow-up in 26 cases, Henoch-Schonlein purpura in 7, and children in 9. In total, 60 patients with normal renal function were enrolled in this study. Thirty-three of them presented with mild-moderate proteinuria and 27 cases with heavy proteinuria. The heavy proteinuria group shared the similar clinical features with the mild-moderate proteinuria group, but these patients had a more advanced histological changes (i.e. grades III and IV) and higher mean diastolic blood pressure. Among these 27 patients, 8 were treated with steroids, 8 with ACEIs/ARBs, and 6 with combined therapy (i.e. steroids plus ACEIs/ARBs). And the other five patients were excluded due to poor compliance, including one in steroids group, one in ACEIs/ARBs group, and three in combined therapy group. After a follow-up of 40.3±31.9 months, we found that daily protein loss decreased 38% in steroids alone group (P＜0.05), 35% in ACEIs/ARBs therapy group (P＞0.05), and 65% in combined therapy group (P＜0.01). Although there were no significant differences of proteinuria reduction among the three subgroups of IgAN patients with heavy proteinuria, there was no non-responder in combined therapy group. Prospective clinical trials are mandatory to prove the efficacy of steroids plus ACEI or ARB therapy in IgA patients with heavy proteinuria.
Systemic lupus erythematosus (SLE) is characterized by the production of pathogenic autoantibodies and the deposition of immune complexes in various organs, whereas minimal change disease (MCD) is characterized by normal or only mild mesangial cell proliferation without deposition of immune complexes. Although these two diseases are so different, they all share the same characteristics of dysregulation of T-lymphocyte and the appearance of low T4/T8 ratio. In this report, we present two cases who had (suspected) SLE developed minimal change NS (MCNS). Case 1 was diagnosed to have SLE, which fulfilled 5 revised SLE criteria of American College of Rheumatology (ACR), but MCD was shown in renal biopsy. Transient renal insufficiency was also noted. Case 2 had NS as well. Renal pathology revealed MCD and tubuloreticular inclusion. High titer of ANA developed later, and the diagnosis of SLE was highly suspected. Both cases got remission from NS after receiving steroid therapy. Since evidence supports that MCNS might be associated or related with SLE, the therapeutic strategy (corticosteroid) is suggested to treat as primary MCNS. Proper recognition of this entity by renal biopsy should be taken into consideration in SLE patient with NS.
Thin basement membrane disease (TBMD) occurs in 5-11% of renal biopsy series, and can be associated with other glomerulopathies (GNs). Data on the prevalence, clinical features, and prognosis of TBMD with other GNs are limited.From June 1990 to May 2001, findings from 658 native kidney biopsies were retrospectively studied. The overall prevalence of TBMD was 7.9% (52 of 658). The mean glomerular basement membrane (GBM) thickness was 206 +/- 30 nm. Clinicopathological features were compared for patients with TBMD only (n = 14) and in those with TBMD and GN (n = 38). Focal segmental glomerulosclerosis, mesangial proliferative GN, and minimal change disease were the most common GNs associated with TBMD. After a mean follow-up period of 44.9 +/- 42.5 months, the group who only had TBMD revealed a relatively benign disease with microscopic haematuria and trivial proteinuria, a low prevalence of hypertension, and no renal progression. In the group who had both TBMD and GN, heavy proteinuria (6.1 +/- 5.2 g/day), hypoalbuminaemia (26 +/- 12 g/L) and renal insufficiency (76 +/- 25 mL/min) might develop.We suggested that the TBMD is a developmental abnormality of little or no significance and that it is the underlying associated GN rather than TBMD, which has the relevance to the outcome of renal disease.
Abstract Serum creatinine (SCr) had been considered to be an important predictor of mortality in end‐stage renal disease (ESRD) patients at the start of renal replacement therapy (RRT). However, the data were limited about initially extreme azotemia (EA), exclusively defined as blood urea nitrogen (BUN)≥300 mg/dL, SCr≥30 mg/dL, or both. This retrospective study was conducted to clarify the characteristics and outcome in our EA patients. We had 1682 new ESRD patients from July 1988 to December 1996. With frequency match for age, gender, and starting RRT in the same period, 20 EA patients and 60 controls were included. Fifty percent of our EA patients had unknown etiology. The EA patients had significantly lower prevalence of underlying diabetic nephropathy, and comorbid hypertension. All the EA patients had late referral to nephrologists within 4 weeks before the initiation of RRT, and 90% of them had taken Chinese herbals. The EA group had significantly higher BUN, SCr, and iron storage as well as a higher prevalence of severe anemia, hyperkalemia, hypocalcemia, and acidemia. However, the similar prevalence of cardiomegaly and left ventricular hypertrophy as well as the similar early mortality rate and long‐term survival were noted. Age over 40 years, comorbid diabetes mellitus, and hypoalbuminemia were independent predictors of poor survival. Our EA patients had different initial presentations from other uremic ones at the start of RRT. However, the short‐term and long‐term mortality rates were similar. The lower prevalence of underlying diabetic nephropathy and comorbid hypertension among the EA patients might contribute to their fair outcome.
Dialysate leakage is one of the most common and important complications is continuous ambulatory peritoneal dialysis (CAPD). However, there is limited discussion about the comolications resulting from leakage of peritoneal effluent caused by a defect of the peritoneal catheter (i.e., a catheter break). We herein report on a uremic patient who received CAPD therapy for 2 years and was bothered with dialysate leakage and ensuing peritonitis, The location of leakage could not be resolved by Tc-99m MAA peritoneal scintigraphy or abdominal computed tomography. The peritoneal catheter was eventually removed, disclosing a large, irregular-edged hole (not indicative of having been trauma-induced) near the external cuff of the intercuff segment of the Tenckhoff catheter, The patient suffered from severe peritonitis with mixed pathogens, and the therapeutic response was poor, Our results suggest that once the CAPD patient is exposed to dialysate leakage and develops unresponsive peritonitis, early removal of the PD catheter should be mandatory in order to avoid a poor outcome.
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